Echocardiographic AMYLI score in systemic light-chain amyloidosis: Clinical relevance and risk stratification

The AMYLoidosis Index (AMYLI) is widely used for screening cardiac amyloidosis, but its relationship with amyloid burden and prognostic value is unclear.

This single-center, prospective, observational study was conducted from November 2011 to September 2023. Consecutive patients with newly-diagnosed biopsy-proven light-chain (AL) amyloidosis patients who underwent both echocardiography and cardiac magnetic resonance (CMR) were enrolled. The AMYLI score was calculated as the product of relative wall thickness (RWT) and E/e' via transthoracic echocardiography.

A total of 263 AL amyloidosis patients (mean age 58.84 ± 9.63 years, 158 [60.1 %] males) were included in the study. The AMYLI score showed a moderately positive correlation with CMR-derived amyloid burden (extracellular volume; r = 0.57, P < 0.001). During a median follow-up of 42 (interquartile range: 35-49) months, 3 patients were lost to follow-up, and 171 patients died. An AMYLI score ≥ 7.85 strongly predicted all-cause mortality (HR 2.80, 95 % CI: 1.80-4.35, P < 0.001) and remained an independent prognostic factor after adjusting for clinical, biochemical, echocardiographic, CMR imaging-related, and therapeutic factors. AMYLI score ≥ 7.85 added incremental prognostic value to conventional clinical and imaging risk factors.

The AMYLI score is a reliable indicator of amyloid burden in patients with AL amyloidosis and independent prognostic factor, offering an alternative and convenient echocardiography-based imaging marker for the risk stratification of AL amyloidosis patients.

Chinese Clinical Trial Registry, ChiCTR1900024764 by the ethics committee of West China Hospital, Sichuan University.